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Record W3109013868 · doi:10.1002/acn3.51249

Brain volumetric deficits in <i>MAPT</i> mutation carriers: a multisite study

2020· article· en· W3109013868 on OpenAlexafffund
Stephanie A. Chu, Taru Flagan, Adam M. Staffaroni, Lize C. Jiskoot, Jersey Deng, Salvatore Spina, Liwen Zhang, Virginia E. Sturm, Jennifer S. Yokoyama, William W. Seeley, Janne M. Papma, Dan Geschwind, Howard J. Rosen, Bradley F. Boeve, Adam L. Boxer, Hilary W. Heuer, Leah K. Forsberg, Danielle Brushaber, Murray Grossman, Giovanni Coppola, Bradford C. Dickerson, Yvette Bordelon, Kelley Faber, Howard Feldman, Julie A. Fields, Jamie Fong, Tatiana Foroud, Ralitza H. Gavrilova, Nupur Ghoshal, Neill R. Graff‐Radford, Ging‐Yuek Robin Hsiung, Edward D. Huey, David J. Irwin, Kejal Kantarci, Daniel Kaufer, Anna M. Karydas, David S. Knopman, John Kornak, Joel H. Kramer, Walter A. Kukull, Maria I. Lapid, Irene Litvan, Ian R. Mackenzie, Mario F. Mendez, Bruce L. Miller, Chiadi U. Onyike, Alexander Pantelyat, Rosa Rademakers, Eliana Marisa Ramos, Erik D. Roberson, Maria Carmela Tartaglia, Nadine Tatton, Arthur W. Toga, Ashley Vetor, Sandra Weıntraub, Bonnie Wong, Zbigniew K. Wszołek, John C. van Swieten, Suzee E. Lee

Bibliographic record

VenueAnnals of Clinical and Translational Neurology · 2020
Typearticle
Languageen
FieldMedicine
TopicAmyotrophic Lateral Sclerosis Research
Canadian institutionsOccupational Cancer Research CentreUniversity of TorontoUniversity of British Columbia
FundersNational Center for Advancing Translational SciencesNational Institute of Neurological Disorders and StrokeNational Institute of Mental HealthNational Institute on AgingCanadian Institutes of Health ResearchParkinsonfondenUniversity of California, San DiegoGenentechAllerganNational Institutes of HealthPfizerBiogenAlzheimer NederlandStichting DioraphteEisaiPuma BiotechnologyNovartisNederlandse Organisatie voor Wetenschappelijk OnderzoekRocheJanssen PharmaceuticalsErasmus Universiteit RotterdamBrightFocus FoundationEli Lilly and CompanyBristol-Myers SquibbParkinson Study GroupAbbVieTauRx PharmaceuticalsSol Goldman Charitable TrustAlzheimer's AssociationZonMwMichael J. Fox Foundation for Parkinson's Research
KeywordsFrontotemporal dementiaMedicineWhite matterAtrophyParkinsonismPathologyUncinate fasciculusDementiaFrontotemporal lobar degenerationMagnetic resonance imagingDiseaseFractional anisotropyRadiology

Abstract

fetched live from OpenAlex

OBJECTIVE: MAPT mutations typically cause behavioral variant frontotemporal dementia with or without parkinsonism. Previous studies have shown that symptomatic MAPT mutation carriers have frontotemporal atrophy, yet studies have shown mixed results as to whether presymptomatic carriers have low gray matter volumes. To elucidate whether presymptomatic carriers have lower structural brain volumes within regions atrophied during the symptomatic phase, we studied a large cohort of MAPT mutation carriers using a voxelwise approach. METHODS: We studied 22 symptomatic carriers (age 54.7 ± 9.1, 13 female) and 43 presymptomatic carriers (age 39.2 ± 10.4, 21 female). Symptomatic carriers' clinical syndromes included: behavioral variant frontotemporal dementia (18), an amnestic dementia syndrome (2), Parkinson's disease (1), and mild cognitive impairment (1). We performed voxel-based morphometry on T1 images and assessed brain volumetrics by clinical subgroup, age, and mutation subtype. RESULTS: Symptomatic carriers showed gray matter atrophy in bilateral frontotemporal cortex, insula, and striatum, and white matter atrophy in bilateral corpus callosum and uncinate fasciculus. Approximately 20% of presymptomatic carriers had low gray matter volumes in bilateral hippocampus, amygdala, and lateral temporal cortex. Within these regions, low gray matter volumes emerged in a subset of presymptomatic carriers as early as their thirties. Low white matter volumes arose infrequently among presymptomatic carriers. INTERPRETATION: A subset of presymptomatic MAPT mutation carriers showed low volumes in mesial temporal lobe, the region ubiquitously atrophied in all symptomatic carriers. With each decade of age, an increasing percentage of presymptomatic carriers showed low mesial temporal volume, suggestive of early neurodegeneration.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.001
metaresearch head score (Gemma)0.002
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.003
Threshold uncertainty score0.006

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0010.002
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.000
Science and technology studies0.0010.000
Scholarly communication0.0000.000
Open science0.0000.001
Research integrity0.0010.000
Insufficient payload (model declined to judge)0.0010.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.224
GPT teacher head0.448
Teacher spread0.224 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

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Citations35
Published2020
Admission routes2
Has abstractyes

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